Rev. Bras. Hematol. Hemoter. vol.34 número6

408

Scientiic Comments

Rev Bras Hematol Hemoter. 2012;34(6):401-10

Involvement of the cerebrospinal luid cells in children with acute lymphoblastic leukemia:

prognostic implications

Carlos Alberto Scrideli

Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo - USP, São Paulo, SP, Brazil

Conlict-of-interest disclosure:

The author declares no competing inancial interest

Submitted: 11/13/2012 Accepted: 11/14/2012

Corresponding author:

Carlos Alberto Scrideli

Departamento de Puericultura e Pediatria. Faculdade de Medicina de Ribeirão Preto da Universidade de São Paulo, FMRP-USP Av. Bandeirantes, 3900

14049-900 Ribeirão Preto, SP, Brazil Phone: 55 16 3602-2672

scrideli@fmrp.usp.br

www.rbhh.org or www.scielo.br/rbhh

DOI: 10.5581/1516-8484.20120101

In this issue of the

Revista Brasileira de Hematologia e Hemoterapia

, Cancela et al.

analyzed the incidence and the risk factors for central nervous system (CNS) relapse in children

and adolescents with acute lymphoblastic leukemia (ALL) treated using the GBTLI-ALL 99

protocol and found that a leukocyte count > 50 x 10

/L at diagnosis was the only signiicant

factor associated with a high incidence of CNS relapse.

Leukemic iniltration of the CNS is deined as ive or more leukocytes/mm

_{and blast}

cells in cerebrospinal luid (CSF) or cranial nerve palsy

_{. It is a well-established prognostic}

factor in children with ALL

_{. The cumulative incidence of CNS relapse (combined or}

isolated) presently varies from 2.6 to 9.5%

_{. No clinical or morphological CNS involvement}

is evident at diagnosis in about 60% of patients who eventually develop CNS relapse

_.

Similar to systemic chemotherapy in the treatment of systemic ALL, there is a growing

concern to identify factors associated with increased risk of leukemia iniltration of the CNS

and to consequently adapt the protocol aimed at the treatment and prophylaxis of neurological

involvement

_{. Several risk factors have been associated with a higher incidence of initial}

involvement or relapse in CNS, such as high risk genetic abnormalities [t(9;22) and mixed lineage

leukemia (MLL) rearrangements], T-lineage ALL and high peripheral leukemic-cell burden

_.

Some studies have also demonstrated that patients with any identiiable blast cells in CSF,

or CSF contamination by blastic cells during traumatic lumbar puncture, present an increased

risk of CNS relapse

_{. Others however have not found this association}

_{. Conventional}

cytological analysis has proved useful, but the analysis of cells in CSF, especially with

low cell counts, is more dificult than is widely admitted and it is not always conclusive

_.

Molecular involvement detected by more sensitive and speciic techniques such as PCR and

direct sequencing has been shown in around 45% of pediatric patients; the prognostic impact

of this molecular involvement seems to be dependent of the intensity of treatment

_{. It is}

possible that morphological involvement of the CNS would represent one of the extremes of a

clinical spectrum ranging from gross to minimal residual disease involvement of the CNS and

that molecular involvement could relect biologically more aggressive disease.

The explanation for these discrepancies is probably related to the eficacy of systemic

and CNS-directed therapy in different treatment regimens, suggesting that the poor prognosis

associated with these variables can be overcome by more effective therapy

_.

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